Wegener's granulomatosis: a new entity in the growing differential diagnosis of Degos' disease

Summary Wegener’s granulomatosis (WG) is a multisystemic vasculitis, with skin involvement in 14% of cases and with palpable purpura, subcutaneous nodules and necrotic papules as the common features.1 We present a patient diagnosed with WG who had multiple whitish papules similar to those of malignant atrophic papulosis (Degos’ disease), which appeared during a flare of his disease. Lesions of malignant atrophic papulosis are said to be pathognomonic; nevertheless, various diseases with similar clinical lesions have been described. To our knowledge, this is the first reported case of such lesions in a patient with WG, and we suggest WG should be included in the differential diagnosis of Degos’ disease.